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难治性贫血和伴有多系发育异常的难治性血细胞减少症中出现外周血原始细胞预示着不良预后。

Presence of peripheral blasts in refractory anemia and refractory cytopenia with multilineage dysplasia predicts an unfavourable outcome.

作者信息

Knipp Sabine, Strupp Corinna, Gattermann Norbert, Hildebrandt Barbara, Schapira Marc, Giagounidis Aristoteles, Aul Carlo, Haas Rainer, Germing Ulrich

机构信息

Department of Haematology, Centre Hospitalier Universitaire Vaudois, Rue du Bugnon 44, 1011 Lausanne, Switzerland.

出版信息

Leuk Res. 2008 Jan;32(1):33-7. doi: 10.1016/j.leukres.2007.02.021. Epub 2007 Apr 6.

Abstract

The World Health Organization (WHO) assigns myelodysplastic syndrome (MDS) to RA/RCMD/RARS/RSCM/5q- syndrome, if medullary blasts are <5% and peripheral blast (PB) count < or =1%. In 1103 patients with these diagnoses, we analysed survival and risk of AML evolution depending on the presence of PB. Median survival in the group with 1% PB (n=74) was significantly lower as compared to those without PB (20 versus 47 months, p<0.00005). Cumulative risk of AML was significantly higher in patients showing PB (p<0.00005). Median survival of patients with PB was not different from that of RAEB I. We therefore propose to consider patients with PB, regardless of medullary blast, as RAEB I.

摘要

如果骨髓原始细胞小于5%且外周血原始细胞(PB)计数小于或等于1%,世界卫生组织(WHO)将骨髓增生异常综合征(MDS)归类为RA/RCMD/RARS/RSCM/5q-综合征。在1103例诊断为此类疾病的患者中,我们根据PB的存在情况分析了生存情况及急性髓系白血病(AML)进展风险。PB为1%的组(n = 74)的中位生存期与无PB的组相比显著更低(分别为20个月和47个月,p<0.00005)。有PB的患者发生AML的累积风险显著更高(p<0.00005)。有PB的患者的中位生存期与RAEB I患者的中位生存期无差异。因此,我们建议将有PB的患者,无论骨髓原始细胞情况如何,视为RAEB I。

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